Introduction: Duodenopancreatic neuroendocrine tumors (pNETs) in multiple endocrine neoplasia type 1 (MEN 1) are the most important cause of MEN 1-related death. Surgery is the only curative treatment, but controversy exists on the optimal strategy. Recent guidelines on pNETs have limited recommendations specific for MEN 1. Therefore, a Dutch multidisciplinary consensus meeting was organized.

Aim(s): To achieve a national consensus on timing and type of initial surgery for pNETs in MEN 1.

Materials and methods: (1) systematic review of literature (2) presentation of case-vignettes and discussion of different scenarios (3) presenting and discussing conclusions to come to a national consensus statement.

Further abstracts you may be interested in

Introduction: Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) expressing somatostatin receptors may be treated with somatostatin analogues (SSAs). Selection criteria are a positive Octreoscan® or a >50% hormone level decrease after octreotide s.c. injection (octreotide test) (OT). Plasma chromogranin A (CgA) is the best general GEP-NET marker, but data on CgA response to OT are scant.

Introduction: Pancreatic endocrine tumors (pNETs) are rare but are among the most common neuroendocrine neoplasms of the abdomen. At diagnosis, many of them are already advanced and difficult to treat.

Introduction: Neuroendocrine tumors (NET) constitute a heterogenous group of neoplasms. The development of Gallium-68-labeled somatostatin analogues, such as DOTA-NOC or DOTA-TOC, PET/CT have dramatically improved the diagnosis of neuroendocrine tumors. Surgery remains the treatment of choice for localized disease as well as metastatic disease. The aims of surgery are: improvement of symptoms, reduction of tumor mass / burden and to give better quality of life to patients. Recurrent laparotomies often lead to multiple adhesions and altered anatomy. So it is increasingly difficult for imaging physicians and surgeons to separate non-malignant from malignant tissue.

Introduction: Neuroendocrine tumors of the pancreas are rare, highly vascularized tumors. Endoglin, a Transforming Growth Factor-β co-receptor, is a marker for angiogenic endothelial cells. Angiogenesis is required for tumor progression and the development of metastases. Recently, endoglin expression was found to be a prognostic marker in pancreatic carcinomas. However, the role of endoglin in neuroendocrine pancreatic tumors has so far not been studied.

Introduction: Gastric endocrine tumors (GET) are increasingly recognized due to expanding indications of upper gastrointestinal endoscopy. Often silent and benign, GET may also be aggressive when sporadic and may sometimes mimic the course of gastric adenocarcinoma. Current incidence of GETs is estimated at around 8% of digestive endocrine tumors. Yearly age-adjusted incidence is around 0.2 per population of 100,000. Gastric carcinoids (ECLomas) develop from gastric enterochromaffin-like cells (ECL cells) in response to chronically elevated gastrin. Type 1 tumors (ECLomas in the course of atrophic gastritis) may occur in conditions of achlorhydria secondary to auto-immune atrophic fundic gastritis. It occurs mostly in women and they are non-functioning tumors, typically found during upper GI endoscopy performed for dyspepsia. ECLomas present frequently as multiple polyps, usually < 1 cm in diameter in the gastric fundus. Type 1 tumors are almost exclusively benign lesions with little risk of deep invasion of the gastric parietal wall. The neoplastic ECL cells become progressively dedifferentiated with an increasing number of Ki-67 immunoreactive (IR) cell nuclei. In addition, there is a substantial decrease in argynophil and IR NE cells that can be visualized by conventional methods. ECLomas secondary to hypergastrinemia should be closely followed for signs of clinical and histopathological tumor progression. Such ECLomas deserve early, active, radical surgical treatment.
Traditionally, gastric carcinoid type 1 (GCA1s) are endoscopically or surgically removed, depending on the number, appearance and size of the tumors. Antrectomy, with surgical excision of the majority of the G cells, is thought to facilitate regression of these tumors by removing the source of excessive gastrin secretion; however, the long-term benefits of antrectomy still remain uncertain. Although proton pump inhibitors are effective in reducing hypergastrinemia-induced gastric acid hypersecretion in GCA2, they do not affect ECL-cell hyperplasia, and therefore their role in GCA1 is limited. Moreover, in selected cases, significant reduction of hypergastrinemia does not prevent development of ECL carcinoid, suggesting that, in addition to hypergastrinemia, other pathogenic or genetic factors may be involved. Treatment with somatostatin analogues (SSA) might impede ECL-cell hyperplasia by suppressing gastrin secretion and/or by a direct anti-proliferative effect on ECL cells. Treatment with SSAs in GCA1 leads to a substantial tumor load reduction, with a concomitant decrease of serum gastrin levels. Published data indicate an important anti-proliferative effect of SSA on ECL cells, providing clinical benefit and obviating, at least temporarily, the need for invasive therapies for GCA1. Morphometric studies demonstrated that, while antrectomy specifically decreased the volume of ECL cells versus the total volume of endocrine cells, octreotide reduces the overall endocrine cell volume. Although the number of treated patients is small, it has been suggested that SSA may exert important anti-proliferative effects either directly, by inhibiting ECL-cells proliferation, or indirectly through suppression of gastrin hypersecretion.

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